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Dermatitis Herpetiformis

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Diagnosis and Management of Gluten-Associated Disorders

Abstract

Dermatitis herpetiformis (DH) is a cutaneous manifestation of celiac disease (CeD) causing a pruritic vesicular rash typically involving the elbows, knees, and buttocks. There is a slight male predominance in DH, and it is rare among children.

DH diagnosis relies on the demonstration of pathognomonic granular immunoglobulin (Ig)A deposits in the papillary dermis detected with direct immunofluorescence examination. Circulating antibodies (Abs) against tissue transglutaminase (tTG) support the diagnosis. The treatment of choice is strict, life-long adherence to a gluten-free diet (GFD). In addition, due to a slow effect of dietary treatment on the rash, patients with severe skin symptoms should additionally be treated with oral dapsone.

The risk of lymphoma is increased in DH, but nonetheless the prognosis of GFD-treated DH seems to be excellent. In fact, the all-cause mortality rate in DH has shown to be lower than that of the general population, unlike the increased mortality associated with CeD.

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Authors and Affiliations

  1. Celiac Disease Research Center, Faculty of Medicine and Health Technology, Tampere University, Tampere, Finland

    Teea Salmi & Kaisa Hervonen

  2. Department of Dermatology, Tampere University Hospital, Tampere, Finland

    Teea Salmi & Kaisa Hervonen

Authors
  1. Teea Salmi
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  2. Kaisa Hervonen
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Correspondence to Teea Salmi .

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Editors and Affiliations

  1. David Geffen School of Medicine at UCLA, University of California Los Angeles (UCLA) Celiac Disease Program, Los Angeles, CA, USA

    Guy A. Weiss

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Salmi, T., Hervonen, K. (2021). Dermatitis Herpetiformis. In: Weiss, G.A. (eds) Diagnosis and Management of Gluten-Associated Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-56722-4_2

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  • DOI: https://doi.org/10.1007/978-3-030-56722-4_2

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-56721-7

  • Online ISBN: 978-3-030-56722-4

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